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Multidrug-resistant nephrotic syndrome

MedGen UID:
868440
•Concept ID:
C4022834
•
Disease or Syndrome
HPO: HP:0012589

Definition

A form of nephrotic syndrome that does not respond to any immunosuppresive treatment. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMultidrug-resistant nephrotic syndrome

Professional guidelines

PubMed

Ofatumumab treatment for nephrotic syndrome recurrence after pediatric renal transplantation.
Bernard J, Lalieve F, Sarlat J, Perrin J, Dehoux L, Boyer O, Godron-Dubrasquet A, Harambat J, Decramer S, Caillez M, Bruel A, Allain-Launay E, Dantal J, Roussey G
Pediatr Nephrol 2020 Aug;35(8):1499-1506. Epub 2020 Apr 18 doi: 10.1007/s00467-020-04567-7. PMID: 32306087

Recent clinical studies

Etiology

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Low-dose ofatumumab for multidrug-resistant nephrotic syndrome in children: a randomized placebo-controlled trial.
Ravani P, Pisani I, Bodria M, Caridi G, Degl'Innocenti ML, Ghiggeri GM
Pediatr Nephrol 2020 Jun;35(6):997-1003. Epub 2020 Jan 28 doi: 10.1007/s00467-020-04481-y. PMID: 31993781
Remission of proteinuria in multidrug-resistant idiopathic nephrotic syndrome following immunoglobulin immunoadsorption.
Nattes E, Karaa D, Dehoux L, Peuchmaur M, Kwon T, Deschênes G
Acta Paediatr 2019 Apr;108(4):757-762. Epub 2018 Oct 15 doi: 10.1111/apa.14582. PMID: 30230027

Diagnosis

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.
Drovandi S, Lipska-Ziętkiewicz BS, Ozaltin F, Emma F, Gulhan B, Boyer O, Trautmann A, Xu H, Shen Q, Rao J, Riedhammer KM, Heemann U, Hoefele J, Stenton SL, Tsygin AN, Ng KH, Fomina S, Benetti E, Aurelle M, Prikhodina L, Schreuder MF, Tabatabaeifar M, Jankowski M, Baiko S, Mao J, Feng C, Liu C, Sun S, Deng F, Wang X, Clavé S, Stańczyk M, Bałasz-Chmielewska I, Fila M, Durkan AM, Levart TK, Dursun I, Esfandiar N, Haas D, Bjerre A, Anarat A, Benz MR, Talebi S, Hooman N, Ariceta G; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium, Schaefer F
Kidney Int 2022 Sep;102(3):604-612. Epub 2022 May 25 doi: 10.1016/j.kint.2022.04.029. PMID: 35643375

Therapy

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.
Drovandi S, Lipska-Ziętkiewicz BS, Ozaltin F, Emma F, Gulhan B, Boyer O, Trautmann A, Xu H, Shen Q, Rao J, Riedhammer KM, Heemann U, Hoefele J, Stenton SL, Tsygin AN, Ng KH, Fomina S, Benetti E, Aurelle M, Prikhodina L, Schreuder MF, Tabatabaeifar M, Jankowski M, Baiko S, Mao J, Feng C, Liu C, Sun S, Deng F, Wang X, Clavé S, Stańczyk M, Bałasz-Chmielewska I, Fila M, Durkan AM, Levart TK, Dursun I, Esfandiar N, Haas D, Bjerre A, Anarat A, Benz MR, Talebi S, Hooman N, Ariceta G; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium, Schaefer F
Kidney Int 2022 Sep;102(3):604-612. Epub 2022 May 25 doi: 10.1016/j.kint.2022.04.029. PMID: 35643375
Low-dose ofatumumab for multidrug-resistant nephrotic syndrome in children: a randomized placebo-controlled trial.
Ravani P, Pisani I, Bodria M, Caridi G, Degl'Innocenti ML, Ghiggeri GM
Pediatr Nephrol 2020 Jun;35(6):997-1003. Epub 2020 Jan 28 doi: 10.1007/s00467-020-04481-y. PMID: 31993781
Mycophenolate mofetil in children with multidrug-resistant nephrotic syndrome.
Bayazit AK, Noyan A, Cengiz N, Anarat A
Clin Nephrol 2004 Jan;61(1):25-9. doi: 10.5414/cnp61025. PMID: 14964454

Prognosis

Rituximab in combination with cyclosporine and steroid pulse therapy for childhood-onset multidrug-resistant nephrotic syndrome: a multicenter single-arm clinical trial (JSKDC11 trial).
Nozu K, Sako M, Tanaka S, Kano Y, Ohwada Y, Morohashi T, Hamada R, Ohtsuka Y, Oka M, Kamei K, Inaba A, Ito S, Sakai T, Kaito H, Shima Y, Ishikura K, Nakamura H, Nakanishi K, Horinouchi T, Konishi A, Omori T, Iijima K
Clin Exp Nephrol 2024 Apr;28(4):337-348. Epub 2023 Nov 27 doi: 10.1007/s10157-023-02431-0. PMID: 38010466Free PMC Article
Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779

Clinical prediction guides

Childhood nephrotic syndrome.
Vivarelli M, Gibson K, Sinha A, Boyer O
Lancet 2023 Sep 2;402(10404):809-824. doi: 10.1016/S0140-6736(23)01051-6. PMID: 37659779
Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.
Drovandi S, Lipska-Ziętkiewicz BS, Ozaltin F, Emma F, Gulhan B, Boyer O, Trautmann A, Xu H, Shen Q, Rao J, Riedhammer KM, Heemann U, Hoefele J, Stenton SL, Tsygin AN, Ng KH, Fomina S, Benetti E, Aurelle M, Prikhodina L, Schreuder MF, Tabatabaeifar M, Jankowski M, Baiko S, Mao J, Feng C, Liu C, Sun S, Deng F, Wang X, Clavé S, Stańczyk M, Bałasz-Chmielewska I, Fila M, Durkan AM, Levart TK, Dursun I, Esfandiar N, Haas D, Bjerre A, Anarat A, Benz MR, Talebi S, Hooman N, Ariceta G; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium, Schaefer F
Kidney Int 2022 Sep;102(3):604-612. Epub 2022 May 25 doi: 10.1016/j.kint.2022.04.029. PMID: 35643375
Mycophenolate mofetil in children with multidrug-resistant nephrotic syndrome.
Bayazit AK, Noyan A, Cengiz N, Anarat A
Clin Nephrol 2004 Jan;61(1):25-9. doi: 10.5414/cnp61025. PMID: 14964454

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